1601 NEUROPHYSIOLOGICAL, PSYCHOLOGICAL, AND NUTRITIONAL INVESTIGATIONS DURING DISCONTINUATION OF THE PHENYL-ALANINE-RESTRICTED DIET IN CHILDREN WITH CLASSICAL PHENYLKETONURIA (PKU)

Abstract

Children with classical PKU had been maintained on a lowphenylalanine diet from the first few weeks of life until their 5th birthday when the phenylalanine-restricted diet was stepwise changed to a regular diet. Physical and neurological examinations, nutritional evaluations, anthropometric measurements, and psychological assessments were done at 4, 4½, 5, and 6 years of age. In addition, neurophysiological investigations including somatosensory, auditory and visual evoked responses were obtained at 4 years 10 months, 5 years, 5 years 2 months, and 5 years 6 months.

Analysis of relationships among the study variables revealed that children who had adequate dietary control and who were of average or above average intelligence displayed normal somatosensory evoked potentials, whereas those children with less adequate dietary control and lower IQ scores had atypical somatosensory evoked potential waveforms. Yet, these multiple assessments did not uncover any significant changes when pre-diet termination data were compared with post-diet termination results.

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